Mitochondrial amidoxime reducing component 1

Mitochondrial amidoxime-reducing component 1 (also known as MOCO sulphurase C-terminal domain containing 1, MOSC1 or MARC1) is a mammalian molybdenum-containing enzyme. It is located in the outer mitochondrial membrane and consists of a N-terminal mitochondrial signal domain facing the inter-membrane space, a transmembrane domain, and a C-terminal catalytic domain facing the cytosol.^[5] In humans it is encoded by the MOSC1 gene.^[6][7]

MTARC1
Identifiers
Aliases	MTARC1, MOSC1, mitochondrial amidoxime reducing component 1, MARC1
External IDs	OMIM: 614126; MGI: 1913362; HomoloGene: 129604; GeneCards: MTARC1; OMA:MTARC1 - orthologs
Gene location (Human) Chr. Chromosome 1 (human)[1] Band 1q41 Start 220,786,352 bp[1] End 220,819,659 bp[1]
Gene location (Mouse) Chr. Chromosome 1 (mouse)[2] Band 1|1 H5 Start 184,518,964 bp[2] End 184,543,510 bp[2]
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in adipose tissue abdominal fat right lobe of liver right lobe of thyroid gland subcutaneous adipose tissue left lobe of thyroid gland monocyte mucosa of transverse colon parotid gland ventricular zone Top expressed in left lobe of liver yolk sac white adipose tissue sexually immature organism mammary gland lumbar subsegment of spinal cord lumbar spinal ganglion brown adipose tissue subcutaneous adipose tissue tunica adventitia of aorta More reference expression data BioGPS n/a
Gene ontology Molecular function molybdopterin cofactor binding molybdenum ion binding oxidoreductase activity pyridoxal phosphate binding nitrate reductase activity catalytic activity Cellular component integral component of membrane membrane mitochondrion mitochondrial outer membrane Biological process detoxification of nitrogen compound nitrate metabolic process xenobiotic metabolic process metabolism Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 64757 66112 Ensembl ENSG00000186205 ENSMUSG00000026621 UniProt Q5VT66 Q9CW42 RefSeq (mRNA) NM_022746 NM_001081361 NM_001290273 RefSeq (protein) NP_073583 NP_001277202 Location (UCSC) Chr 1: 220.79 – 220.82 Mb Chr 1: 184.52 – 184.54 Mb PubMed search [3] [4]
Wikidata
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MOCO stands for molybdenum cofactor.

MOSC1 has been reported to reduce amidoximes to amidines.^[8][9]

Genetic variation in MARC1 has been reported to be associated with lower blood cholesterol levels, blood liver enzyme levels, reduced liver fat and protection from cirrhosis suggesting that MARC1 deficiency may protect against liver disease.^[10] A genome-wide association study involving subjects from the UK Biobank further established as association of alcoholic-related liver disease.^[11]

See also

• MOCOS